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A case of Marfan syndrome with massive haemoptysis from collaterals of the lateral thoracic artery.

Authors :
Yabuuchi, Yuki
Goto, Hitomi
Nonaka, Mizu
Tachi, Hiroaki
Akiyama, Tatsuya
Arai, Naoki
Ishikawa, Hiroaki
Hyodo, Kentaro
Nemoto, Kenji
Miura, Yukiko
Hase, Isano
Usui, Shingo
Oh-ishi, Shuji
Hayashihara, Kenji
Saito, Takefumi
Chonan, Tatsuya
Source :
BMC Pulmonary Medicine; 1/8/2020, Vol. 20 Issue 1, p1-5, 5p, 1 Color Photograph, 4 Black and White Photographs
Publication Year :
2020

Abstract

<bold>Background: </bold>Marfan Syndrome (MFS) is a heritable connective tissue disorder with a high degree of clinical variability including respiratory diseases; a rare case of MFS with massive intrathoracic bleeding has been reported recently.<bold>Case Presentation: </bold>A 32-year-old man who had been diagnosed with MFS underwent a Bentall operation with artificial valve replacement for aortic dissection and regurgitation of an aortic valve in 2012. Warfarin was started postoperatively, and the dosage was gradually increased until 2017, when the patient was transported to our hospital due to sudden massive haemoptysis. Computed tomography (CT) with a maximum intensity projection (MIP) revealed several giant pulmonary cysts with fluid levels in the apex of the right lung with an abnormal vessel from the right subclavian artery. Transcatheter arterial embolization was performed with angiography and haemostasis was achieved, which suggested that the bleeding vessel was the lateral thoracic artery (LTA) branch. CT taken before the incident indicated thickening of the cystic wall adjacent to the thorax; therefore, it was postulated that the bleeding originated from fragile anastomoses between the LTA and pulmonary or bronchial arteries. It appears that the vessels exhibited inflammation that began postoperatively, which extended to the cysts.<bold>Conclusion: </bold>We experienced a case of MFS with massive haemoptysis from the right LTA. We have to be aware of the possibility that massive haemoptysis could be induced in MFS with inflamed pulmonary cysts. [ABSTRACT FROM AUTHOR]

Details

Language :
English
ISSN :
14712466
Volume :
20
Issue :
1
Database :
Complementary Index
Journal :
BMC Pulmonary Medicine
Publication Type :
Academic Journal
Accession number :
141098546
Full Text :
https://doi.org/10.1186/s12890-019-1033-1